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ALS/MND is a relentlessly progressive neurological disorder culminating in complete dependence on others for all activities of daily living. In Ireland one person dies every five days. ALS/MND has been identified as an illness that is particularly prone to engendering a sense of helplessness and failure which leads to feelings of frustration and de-skilling that can result in health-care professionals avoiding patients. (Skyes 2006 ) The clinical management of ALS/MND is palliative from the time of diagnosis and is focused on symptom control and adjustment to the progressive loss of neurological function with the certainty of early death. As treatments are limited, inevitable decisions regarding accepting or forgoing life-sustaining therapies should be made. The failure to address advance care planning leads to unplanned interventions, particularly mechanical ventilation. Decisions to withhold or withdraw life-prolonging treatments are among the most difficult for patients, carers and health-care professionals. Background

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Aims of the Study To identify the attitudes, understandings and experiences of patients with ALS/MND, their carers and their health-care professionals to end-of-life decisions and advance care directives To denote the differences and/or similarities between the experiences of the patients, their carers and their health-care professionals. This study was funded by the Health Research Board.

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Methodology A hermeneutic approach, guided by the philosophy of Hans Georg Gadamer, was the chosen methodology for this study as it illuminates the meaning and understanding of the lived experience of contemplating end-of-life decisions and advance care directives for patients with ALS/MND, their carers and their health-care professionals Purposive sampling was used. The participants selected had experience pertaining to the phenomena under investigation not just an opinion Participants included patients with ALS/MND their carers and their health-care professionals, including nurses, Palliative Care Consultants and Consultant Neurologists

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Inclusion and Exclusion Criteria Inclusion criteria for health-care professionals: All the health-care professionals had experience of the phenomena under investigation. In Hermeneutics it is the lived experience that is required and not just an opinion. Exclusion criteria for health-care professionals: Health-care professionals who had no experience of discussing end-of- life issues and advance care directives with ALS/MND patients.

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Advance Care Directive An Advance Care Directive is a statement made by a competent adult relating to the type and extent of medical treatment he or she would or would not want to undergo in the future should he/she be unable to consent or dissent at that time. (The Irish Council for Bioethics 2007)

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Sample Advance Care Directive To my Family, Doctors, Health Care Team and other persons concerned! Name: Address: Date of Birth: I received a diagnosis of MND/ALS on The objective of this Advance Directive is to spare my family, carers and medical advisers the burden of making difficult decisions on my behalf. I am fully informed regarding diagnosis and prognosis on my MND/ALS. I am also aware of the treatment options available to me. Medical Therapy Decisions: Non-invasive ventilation Gastrostomy insertion Hydration - Nutrition Antibiotic treatment Palliative Care Cardio-pulmonary- resuscitation Invasive ventilation Locus of Care After careful consideration and discussion with my medical advisors and my family I have freely and in sound mind decided with the support of my family that: As my illness progresses I wish my future nursing care and symptom management to be Palliative. I do not wish to have the following treatment interventions: I wish to have to have the following treatment interventions: I am aware that I cannot insist on receiving any particular treatment: I have discussed this document with my General Practitioner: G.P. Signature: Address: Date: Phone number: I reserve the right to revoke this directive at any time. Signature: _________ Witness: _________ Date: _________ Copies of this document are with my GP, solicitor, next of kin and hospital records.

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Advance Care Directives For the patients and carers that participated in this study, contemplating end-of-life decisions and having advance care directives were profound undertakings which were approached in a unique and individualized way. For some patients there are worse things than death: for others long term invasive ventilation may be their preferred choice. The realistic medical options and interventions available will determine and influence these decisions. Johnston 2006 “Advance care planning should be firmly grounded in the values of the individual who, in turn, should understand the consequences of the decisions, both for themselves and their family”. Advance care directives are increasingly recognised as important tools for safeguarding autonomy. However, not all patients wish to make advance care directives and some health-care professionals have difficulties implementing advance care directives.

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Autonomy “Autonomy is not just a status, but a skill, one that must be developed. Health-care interactions rely upon assent, rather than upon genuinely autonomous consent. Throughout most of their medical lives, patients are socialised to be heteronomous, rather than autonomous. At the worst possible time – when life and death consequences are attached to the choices, the paradigm shifts and real consent is sought, even demanded, making an often traumatic situation even harder.” ( C. Myers 2004). “While an individual might want to express the right to self-determination, any decision they make regarding medical treatment will be influenced by the views of third parties, the individual’s doctor, family or friends” (Irish Council for Bioethics 2007). (Irish Council for Bioethics 2007).

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Individualised impact of the illness ALS/MND is a relentlessly progressive fatal illness resulting in a series of losses. It is characterized by progressive decline in motor function culminating in complete dependence on others for all activities of daily living. The impact of ALS/MND is a unique and individualised experience influenced by how the patient and their carer perceive the changes caused by the illness, their previous coping mechanisms, the presence of support structures and the strength of their relationship prior to the diagnosis. For many patients with ALS/MND the shock of the diagnosis is compounded by the relentless progression of the illness. They continued to maintain hope and a positive outlook, considering themselves lucky and blessed and continued to enjoy and reported having a good quality of life, living for today. “As one finds meaning in the present, it is possible for life to be experienced as deeper, richer and more rewarding even while living with physical decline, realizing one’s finitude, the present time becomes more precious” (Lambert 2006)

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Difficulties discussing end-of-life issues for patients and their carers There needs to be an acknowledgement that death, while not imminent, will be the likely outcome of the illness. One of the major problems is trying to plan for something that is abstract: “I live for now... I don’t dwell on it...I think it’s very good to plan your dying...and I’m saying you’re dying as from the time you start on, are on the last hill...I’m on one now but it’s only a slope, but on that last hill, yes it is useful then” Patients are requesting that they remain in control and make decisions about their death, just as they did throughout their lives. For some their end-of-life wish is not to die!

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Burden of being a carer Family caregivers are key figures in ALS/MND care. They usually provide the bulk of support to patients and play a significant and crucial role in clinical decision making process. “The patients are so dependent on their primary caregivers that the couple represents a unique entity, a psychological dyad whose components cannot be considered separately” (Chio et al 2004) There were concerns and anxieties expressed as advance care directives are not legal, would there be difficulties implementing them? Would there be a battle with some health-care professional at the end of a difficult and painful disease process to have the patient’s wishes respected? “I hope the end of his life, will not result in an argument with an official”

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Burden of responsibility for Carers Many of the participants identified the importance of family support throughout the disease trajectory but highlighted the importance of family consensus regarding end-of-life decisions and advance care directives. The carers reported that they had great difficulty discussing end-of-life issues and feared family recriminations if they supported or participated in the patients’ decision to request or refuse treatment. The carers reported their reluctance to revisit E-o-L discussions, once a decision was made, fearing that revisiting these issues would cause upset or that the decisions made would be revoked. Not all patient’s may wish to make ACD and this adds to the burden of responsibility for their carers.

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Difficulties for H-C-P discussing End-of-Life issues The difficulty faced by most health-care professionals in initiating end-of-life discussions with patients with ALS/MND is that there needs to be an acknowledgement by the patient at some level that death, while not imminent, will be the likely outcome of the disease progression. One of the difficulties for H-C-P is to identify the most appropriate time to initiate end-of-life discussions and the importance of having these discussions before the patient loses the ability to communicate verbally. The H-C-P highlighted the importance of knowing the patient, of having a good rapport, of having a trusting relationship and also the importance of having the skills to discuss theses issues sensitively and competently. The H-C-P who participated believed that initiating the discussion, while difficult, was welcomed by most patients and their families.

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Burden of Responsibility for Health-Care Professionals The responsibility of ensuring that their patients are fully informed about the likely disease outcome. That the patients understand the implications of their decisions. That they understand that their decisions may influence the treatments they receive, the services that are available to them and may determine their place of death. Many reported the difficulties they encountered in dealing with different multidisciplinary teams and how the lack of communication and personal beliefs resulted in patients’ wishes not being respected. All the health-care professionals agreed that attendance at the ALS/MND clinic ensured that E-o-L issues and advance care planning would be discussed in a timely and appropriate manner. However, their concerns emanated from their experience that although patients had ACD these were not respected in local hospitals.

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Dilemmas Many health-care professionals believe it appropriate to respect and implement ACD. However, there are some who feel that life should be preserved at all cost. An anaesthetist refused to implement the ACD of a patient with ALS/MND stating that “we don’t polish off patients in the I.T.U. setting” The I.T.U. nurses who said that “if having a baby you would have an expected date of delivery, are we now being asked to have an expected date of death” “When working in an acute setting the philosophy is often guided by technical, scientific and curative treatments, resulting in difficulties for health-care professionals switching to the holistic philosophy of palliative care” Roche-Fahey and Dowling (2008) Health-care professionals have a right to their autonomy and the right to care for individuals without abandoning their own integrity. Many may not know the patient and they may have concerns that the directives may not actually reflect the current situation.

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Palliative Care All the participants identified the importance of including Palliative Care services and how vital their skills are in dealing with the complexities of end-of-life issues. There continues to be a stigma attached to including Palliative Care. Patients believe that their “days are numbered” if referred. Health-care professionals tend to back off, withdrawing their service, believing that if Palliative Care services are involved, there is nothing left to be done. Many believed that the palliative care team should be introduced earlier in the disease trajectory and that their services should be available for symptom management throughout the illness, not just in the terminal phase.

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Legal Issues Some of the participants expressed concern that ACD are not legal: they hoped that legislation would provide clear guidelines and therefore ensure that they would be respected. Others believed that even if they are not legal, by presenting an ACD you are alerting a health-care professional to the expressed wishes of the patient. Amongst the health-care professionals that participated, there was ambivalence regarding the legislation of ACD and a view that a combination of having a verbal directive and having a nominated proxy would work very well. All the participants agreed that their preference was for a disease specific advance care directive.

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Findings of the study There needs to be improved clinical supervision and ongoing educational programmes for health-care professionals who are dealing with the very challenging, emotional and ethical difficulties identified in caring for these vulnerable patients. There needs to be improved communication between the tertiary hospital multidisciplinary teams and the community teams. An individualised approach is required and end-of-life issues should be discussed earlier in the disease process and these issues should be initiated by the health- care professionals. Referral to the ALS/MND centre of excellence may result in timely and appropriate end-of-life and advance care discussions taking place. A sample advance care directive document or a booklet may facilitate discussing end-of-life issues.

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Conclusions A disease specific advance care directive was deemed more appropriate than the generic advance care directive proposed by the Law Reform Commission. There is a need for increased public information and awareness regarding end-of life decisions and advance care directives whereby it does not have to be a sentinel event before these issues are discussed. End-of-life decisions and advance care directives are an extremely important process that emerges in the context of the patient-healthcare professional relationship. It involves much more than completion of a formal advance care directive form or “ticking the box” The discussion of end-of-life may appear to be fraught with difficult decisions and ethical challenges. The potential for conflict may lead health-care professionals to avoid open discussion and advance care planning. Johnston (2006) “believes that informing and guiding patients and their families through the decision making process to a peaceful death should be integral to medical practice”.

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Conclusions This study has illuminated the difficulties and the burden of responsibility faced by health-care professionals and the carers providing end-of-life care for patients with ALS/MND. Patients need to be aware that their autonomy is not absolute, that they cannot insist on receiving specific, unrealistic or illegal treatment nor can they compel their attending health-care professionals to act against their conscience. The difficulty for patients who wish to invoke an advance care directive and have their end-of-life wishes respected is that they are completely dependent on the attending Physician or health-care professional to respect and/or implement their wishes. Despite a hope that the use of ACD’s would ensure that patients’ preferences for their end-of-life wishes would be respected this study has illuminated that the use of ACD’s cannot promise or guarantee the patients a say in their future care.

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Thank You Many thanks to the patients, their carers and the health-care professionals who kindly participated in this research study.